Archives for August 2011

First off, just wanted to share with everyone that Hannah’s “mysterious lump” was just a cyst!!!!   Talk about relief 🙂   Even better, it is almost gone already.

Next week is a big week for Hannah’s educational, therapeutic, and socializational future.   We have her first IEP planning meeting on September 1st.  You know, I just realized that I don’t even know where the meeting is being held — I better call on Monday to find out!

But before that meeting, Hannah has an audiology appt on Monday and then on Tuesday a pre-IEP meeting with the school district nursing supervisor, Ethan and Abby’s new school nurse, and a school district nurse who specializes in trach kids.   I felt very good that they called and wanted this meeting — tells me that they are really wanting to look at Hannah, the person, not Hannah, the “medically fragile kid on paper.”

I’m not sure what to expect at all for this IEP meeting.

Today we went to Ethan and Abby’s new school open house.   I just LOVED all the people there, the kids’ teachers, the support staff, and we even introduced ourselves to the school nurse who will be out to our house next week for the pre-IEP meeting.    They have a ‘strong’ special early education program, but I don’t know if it will be the right fit in their eyes for Hannah.

If Hannah could go to the kids’ school, OMG, that would be a dream come true!   Just thinking about it is getting me too excited — almost too good to be true.   I keep thinking how I would be able to volunteer at the kids’ classes while Hannah is in class, how if Hannah is needing some loving during the day, Abby and Ethan could be right there (boy were they excited about that idea!  “Mommy, I will come give Hannah kisses every day in class!”

Getting ahead of myself here, I know.   Keeping my fingers crossed that what is best for Hannah will work out to be there!

Hannah grabbing the toy Ethan is holding out for her -- see that, she is GRABBING it! She couldn't even control her hand movements a few weeks ago.

We have always been told that Keppra was a very safe drug, “benign” was even one of the words used to describe it by one of Hannah’s former neurologists.   She was put on it just days after her stay began at TCH well over a year ago after her initial tonic-clonic seizure brought on by her breath-holding spell.

When she was discharged last July, she was at 1.0 mL three times a day.   After her “seizure-filled” EEG last November, it was upped to 2.0 mL three times a day.   Hannah’s fine motor and balance problems started the end of December and spiraled from there.

It took a specialist who wasn’t familiar with Gaucher’s to make the connection for us.  Had Dr. Sanger not told us that he doesn’t like to use Keppra and that it shouldn’t be used for kids who are neuro-compromised…along with the fact that he didn’t believe what was seen on the EEG were actual seizures but corticol myoclonus….

It angers me that we didn’t make the connection sooner.

Here we are less than two weeks after started our wean off from Keppra.  We are now at 1.0 mL three times a day after a week of being at 1.5 mL three times a day.   Next Sunday, we will be down to 0.5 mL three times a day.   Our goal is to have her completely off Keppra by her next EEG at the end of September.   (Obviously if we see seizure activity, we will call her neuro immediately and look into another seizure-med).

Ethan and Abby are LOVING the fact that Hannah is starting to be able to interactively play with them again!

SO…..We have seen some amazing changes in Hannah in just the past week or so.

One month ago, Hannah couldn’t balance at all when sitting on her knees (her W-sit).   Her balance was just bad that she would wobble and eventually fall over if we weren’t holding her.

NOW…She is able to sit on her knees BY HERSELF for almost 30 seconds!  Her balance has improved so well already!   Confidence wise, she needs to have us right there to fall on or lean on, but it is so wonderful seeing her sit on my lap without me having to hold her entire back and neck.  I just have to hold her around her waist, and she can sit up and support herself.

One month ago, Hannah’s fine motor skills were almost non-existant.   She could no longer grasp toys, reach out to touch anything, or even play the special needs apps on her iPad.   Because her movements and control were so bad, she was constantly holding her arms and couldn’t do anything else.

NOW…She can reach up and grab her new favorite toy, a ball with lots of grabbing areas, that is lifted a foot above her head!  She actually can locate it and put her arm, albeit slowly and with a lot of effort, up and open her fingers to grab at all!   She lays on her side and grabs it all the time as well.

Today she also was sitting her in Pink Rider (wheelchair stroller) and was able to touch the keys on her lighted musical piano for the first time in many MONTHS!   Again, it was kinda of sloppy and not a direct easy touch, but she worked hard to get her hand there and move it!

So my question is — WHAT THE HELL DID THE KEPPRA DO TO HER?!

If we are seeing this kind of improvement in less than just two weeks, what are we going to see in the next two months?!   Does she even have a seizure disorder at all (did the EEG tech that did that fateful November EEG know what cortical myoclonus even was and how it is NOT a seizure?)?

We haven’t even restarted the Klonopin wean yet!    We had seen a huge personality change when we dropped from 3.0 mL three times a day to 2.0 mL three times a day.   She was no longer lethargic, became her happy charming self again, and showed, most importantly, INTENT to want to do things.

Now with this Keppra and all these changes….UGH!!!!!     What if some of her current issues were just bad med choices and not all Gaucher progression?   Everyone just assumed it was progression (even us), but now seeing this dramatic (to us) improvement over just the past two weeks….UGH!!!!!

A bit less than a week ago, Hannah started developing this red rash on the outside of her left elbow.   It wasn’t hot to touch, inflamed, or pussy.   It didn’t even seem to affect her much.  It was just a rash.

Going back in our heads as to “what has changed” in the past week or so, Daddy and I thought it could have been the addition of the chamomile tea.

The tea has been a godsend in terms of letting her sleep through the night!   She had gone from averaging about 5 hours or so of sleep a night to 8 or 9!

So of course, because it had been working so well, it probably was the tea giving her the rash, because let’s face it — nothing is ever simple with Hannah.

These past three nights we decide against giving her tea to see if the rash would go away.   Sure enough, it definitely has started to dissipate…along with Hannah’s good night sleep.   Each night, without the tea, she only slept 4 or 5 hours.

Heartbreaking.

So we are trying something tonight.   Instead of giving her the 5 or 6 ounces of tea at night, we are going to limit it to just 3 ounces at around 2 am and see if that will allow her to sleep a bit longer without the rash coming back.

If the rash comes back, it is on to plan B…  whatever that may be.

Gosh, I hope we can keep her on the tea and that the rash was just a fluke.   It has worked so well for her sleep!   Keeping my fingers crossed this works.

Two days ago, Hannah’s nurse noticed a little bump under her left armpit when she was giving her a bath.    You have to feel for it, but you can touch it, and it seems to move a bit.   It doesn’t hurt her when you touch it either.

Yesterday morning, this bump was clearly visible without having to look for it.   Still didn’t seem to hurt her, but it was concerning enough to me that I brought her in to our pediatrician in the afternoon.

She commented how it seemed “cystic” in nature, and how it wasn’t inflamed, red, or hot to the touch.  It actually was almost slightly bluish-greyish.   It was about 2 inches lower than where the lymph nodes are located.

All I kept thinking was “when does Hannah get a break?”

Because our pediatrician wasn’t exactly sure what it was, Hannah is scheduled for a left breast ultrasound on Monday to have it checked out.   I’m hoping, hoping, hoping it is something completely benign like a cyst or a blood vessel.  It just has to be!

Monday just can’t get here soon enough!

One of the other issues that was brought up with Dr. Sanger a few days ago was that of Hannah’s lack of sleep.   Including naps, she averages about 8 hours of sleep a day.   Rarely more, sometimes less.

We explained to him that sometimes when she is sleeping and wakes up in the night (just as many people do for a few seconds or so), her movement disorder kicks in and she can’t fall back to sleep.    Also, her movement disorder is so constant that it is also hard for her to initially calm down enough to go to sleep, and it can sometimes take hours (no joke!) before she finally crashes.

Dr. Sanger believes that Hannah’s condition also could start to improve with just the addition of getting more and better sleep.   When a body is in constant motion like hers, it is constantly wearing her out.   Without good sleep, she doesn’t let her body get a chance to rejuvenate energy and muscle.

He wants to consider putting her on a sleep aide such as Lunesta or something.   Even though it isn’t prescribed for kids, he believes it would be safe enough (another thing he is checking with his Pharmacology group).   We have been so afraid of sedatives because we thought her movement disorder was caused by oversedation, but if he is right and it isn’t, this may be just the thing for her!

When we told my parents (whom we were staying with in LA) about this, they suggested why not try chamomile tea.

So that night, we got out my parents tea and gave her some instead of some of her nighttime water.   She was out in less than 60 minutes!   However, she still woke up about 5 hours later.

The next night, she happen to fall asleep on her own at 10 pm, but I gave her some tea instead of her nighttime water at around 1:30 am.   She slept until 6 am!!   She was a little restless at around 1 am before I gave her the tea, but all in all, that was almost an 8-hour stretch of sleep for her!

So tonight, we are going for three nights in a row.   I gave her some tea (2 oz) around 10 pm, and she fell asleep around 11 pm.    I’m going to give her some more at around 2 am (instead of her 2 am water, 3 oz), and I can only hope that she sleeps in again.

I’m not getting my hopes up just yet, but it would be wonderful for her (and me) to not wake up at 3 am in the morning anymore and let her get a REAL sleep!!

Could it really be as simple as chamomile tea?

We met with Dr. Terence Sanger down at Children’s Hospital Los Angeles a few days ago.  We is one of the leading pediatric movement disorder specialists in the country, and we went with an open mind, not expect answers, just hoping for some, well, hope!

It went better than we expected!   It was very clear that he went through Hannah’s paperwork very carefully before our meeting because he asked very specific questions that could only be found in the paperwork.   He spent almost 30 minutes with us (along with his fellow, two residents, and a videographer who tapes all of his clinic visits).

It was a very eye opening visit and gave us a LOT of things to look at and to think about.

• He doesn’t believe that Hannah’s movement disorder was caused by oversedation during her hospitalization.   He believes it would more likely have been caused by a hypoxic brain event during that initial seizure.  He brought up how her CO2 was really high during that time and that her O2 sats in the ambulance were in the 50s.     Her movement disorder was clearly apparent just 7 days after the initial event, 5 days after intubation.   After really thinking about it more, Daddy and I both remember that right after she was admitted, she had a difficult time breathing when she was awake (causing more respiratory distress) and when she was sleeping, she was breathing much better.    Coincidence?
• He is a strong believer against using Keppra for seizure control in children who are already developmentally compromised such as Hannah.    He also is not convinced that she is having subclinical seizures while she is sleeping based on our description but may likely be cortical myoclonus (which is a Gaucher symptom).    Again, after thinking about it more, Hannah’s Keppra was upped in November due to the results of that EEG back then…and her fine motor control and balance started getting worse around Christmastime.   Coincidence?